AB0585 NEURO-BEHÇET’S DISEASE: A TUNISIAN EXPERIENCE
Authors: A. Baya Chatti, S. Skhiri, A. Guiga, W. Ben Yahia, A. Atig, N. Ghannouchi
Journal: Annals of the Rheumatic Diseases (2022)

Background Behçet’s disease (BD) is a systemic inflammatory vasculitis with a wide range of manifestations including neurological involvement. Though neuro-Behçet’s disease (NBD) is relatively uncommon, it encompasses some of the most serious manifestations of BD. Its diagnosis can be challenging, as there is no specific diagnostic test. Objectives Our study aimed to describe epidemiological, clinical and paraclinical characteristics of BD, and compare them between patients with and without NBD. Methods We conducted a retrospective study, including all patients meeting the revised international study group criteria for BD, over an 18-year period. We divided patients into 2 groups, based on the presence or absence of neurological involvement, retained according to the international consensus recommendation criteria for NBD. Epidemiological, clinical and paraclinical features were then analysed and compared between the two groups. Results We obtained a cohort of 77 patients with a gender ratio (M/F) of 1.85 and a mean age at diagnosis of 34.4 years [20-60]. Mucocutaneous involvement inaugurated BD in 81.8% of cases, followed by joint involvement (19.5%), vascular involvement (17.9%), neurological involvement (10.7%) and ocular involvement (2.6%). Mucocutaneous involvement was dominated by oral aphtosis (100%) and genital aphtosis (79.2%) with a mean frequency of episodes per year of 8.3 ± 6.2 and 6.9 ± 5.1 respectively, followed by pseudo-folliculitis (66.2%), erythema nodosum (19.5%), and positive pathergic test (13%). Joint involvement (62.3%) was mainly peripheral (arthralgia in 98% and arthritis in 25%), affecting large joints (85.1%) and bilateral in 78.8% of cases (asymmetric in 48.6%). Axial involvement and sacroiliitis were observed in 14.6% and 10.4% of cases, respectively. Only one patient had a popliteal cyst. Vascular manifestations (36.4%) were venous thrombosis (92.9%), arterial thrombosis (12.9%) and arterial aneurysms (6.5%). Ocular manifestations (35%) were uveitis and vasculitis in 77.8% of patients respectively, with predominance of bilateral lesions (70.4%). Other manifestations were cardiac involvement (5.2%), intestinal involvement (3.4%) and fever (23.4%). One patient developed membranous glomerulonephritis without any other underlying aetiology. Biological inflammatory syndrome was found in 42.9% of patients. Twenty-eight patients developed NBD with 34 outbreaks during follow up. The mean delay from onset of BD was 6.3 years [0-26]. Parenchymal NBD was more frequent (73.5%). Non-parenchymal syndromes (38.2%) were acute meningitis (17.6%), cerebral venous thrombosis (11.8%), pseudotumour cerebri (5.9%) and isolated intracranial hypertension (2.9%). One patient developed peripheral neurological involvement. Headache (59%) and motor deficits (32.4%) were the most common signs. Neurological examination most frequently revealed a pyramidal syndrome (32.3%). Severe forms (modified Rankin scale ≥2) dominated clinical presentations (64.7%). CSF study revealed abnormalities in 10 (35.7%) cases: Increased protein with pleocytosis in 6 cases with lymphocytic predominance (n=3), mixed formula (n=2) and PNN predominance (n=1), decreased glucose levels and increased pressure in 3 cases respectively, and one case of albuminocytological dissociation. The predilection sites of NBD lesions on MRI were brainstem and basal ganglia (35.5%, respectively), periventricular white matter (26.5%), subcortical WM (23.5%), internal capsule (20.6%), centrum semiovale and cerebral cortex (11.8%, respectively) and cerebellum (3%). Comparison between the two groups revealed, in patients with NBD, an older mean age at diagnosis (37.7±11.97 vs 32.3 ±7.14, p=0.023) and a lower frequency of ocular involvement (21.4% in G1 vs 42.9% in G2, p=0.05). We found no statistically significant differences when comparing gender ratio and other clinical and biological features. Conclusion Ocular lesions were more frequent in patients without NBD. Large-scale studies are needed in order to determine markers associated to neurological involvement. Disclosure of Interests None declared